Idiopathic and Systemic Sclerosis-Associated Pulmonary Arterial Hypertension: A Comparison of Demographic, Hemodynamic, and MRI Characteristics and Outcomes

Chest. 2017 Jul;152(1):92-102. doi: 10.1016/j.chest.2017.02.010. Epub 2017 Feb 20.


Background: Previous studies have identified survival in systemic sclerosis (SSc)-associated pulmonary arterial hypertension (SSc-PAH) as being worse than in idiopathic pulmonary arterial hypertension (IPAH). We investigated differences between these conditions by comparing demographic, hemodynamic, and radiological characteristics and outcomes in a large cohort of incident patients.

Methods: Six hundred fifty-one patients diagnosed with IPAH or SSc-associated precapillary pulmonary hypertension were included. Patients with pulmonary disease or two or more risk factors for left heart disease were identified, leaving a primary analysis set of 375 subjects. Subgroup analysis using cardiac magnetic resonance (CMR) imaging was performed.

Results: Median survival was 7.8 years in IPAH and 3 years in SSc-PAH (P < .001). Patients with SSc-PAH were older with less severe hemodynamics but lower gas transfer (diffusing capacity for carbon monoxide [Dlco]). Independent prognostic factors were age, SSc, Dlco, pulmonary artery saturation, and stroke volume. After excluding patients with normal or only mildly elevated resistance, there was no difference in the relationship between pulmonary vascular resistance (PVR) and compliance in IPAH and SSc-PAH. The relationship between mean pulmonary arterial pressure (mPAP) and systolic pulmonary arterial pressure (sPAP) in IPAH was identical to that previously reported (mPAP = 0.61 sPAP + 2 mm Hg). The relationship in SSc-PAH was similar: mPAP = 0.58 sPAP + 2 mm Hg (P value for difference with IPAH = 0.095). The correlation between ventricular mass index assessed at CMR imaging and PVR was stronger in SSc-PAH.

Conclusions: The reasons for poorer outcomes in SSc-PAH are likely to be multifactorial, including but not limited to older age and reduced gas transfer.

Keywords: connective tissue disease; idiopathic; pulmonary hypertension; systemic sclerosis.

Publication types

  • Comparative Study

MeSH terms

  • Adult
  • Age Factors
  • Aged
  • Demography
  • England / epidemiology
  • Familial Primary Pulmonary Hypertension* / diagnosis
  • Familial Primary Pulmonary Hypertension* / epidemiology
  • Familial Primary Pulmonary Hypertension* / physiopathology
  • Female
  • Hemodynamics
  • Humans
  • Hypertension, Pulmonary* / diagnosis
  • Hypertension, Pulmonary* / epidemiology
  • Hypertension, Pulmonary* / etiology
  • Hypertension, Pulmonary* / physiopathology
  • Magnetic Resonance Imaging / methods
  • Male
  • Middle Aged
  • Outcome Assessment, Health Care
  • Respiratory Function Tests / methods
  • Scleroderma, Systemic / complications*
  • Severity of Illness Index
  • Survival Analysis
  • Vascular Resistance